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Congenital melanocytic nevi: update in genetics and management. PURPOSE OF REVIEW: The article intends to review recent updates in the management of congenital melanocytic nevi (CMN) and the evolving genomic landscape that has begun to shed light on the nature of nevogenesis, malignant potential, and possible therapeutic targets for those with melanoma and neurologic involvement. RECENT FINDINGS: CMN are the result of postzygotic somatic mutations involving key proteins in the mitogen-activated protein kinase pathway, primarily NRAS and BRAF. Complications include a spectrum of neurologic findings and development of melanoma. Patients with extracutaneous involvement may be better termed as having 'CMN syndrome'. MRI findings in high risk patients with multiple CMN may best predict clinical outcome. SUMMARY: The management of patients with CMN is complex. Neurologic involvement and melanoma remain the two most devastating complications. New, genetically targeted therapies for patients with complications may be of value, and research for potential therapies is ongoing.